Early-Onset Spinal Deformity in Neurofibromatosis Type 1: Natural History, Treatment, and Imaging Surveillance

Majd Marrache, Krishna V. Suresh, Daniel J. Miller, Steven Hwang, Elizabeth K. Schorry, Jonathan J. Rios, Paul D. Sponseller

Research output: Contribution to journalArticlepeer-review

Abstract

»: Early-onset scoliosis (EOS) or kyphosis is common in patients with neurofibromatosis (NF) and is characterized by rapid progression of deformity. »: Traditional growing rods provide good functional and deformity outcomes in patients with NF and EOS; magnetically controlled growing rods (MCGRs) also provide good deformity correction, although high rates of revision have been reported after their use. »: Among patients with NF type 1 (NF1), morphologic characteristics of the spinal deformity are different in those with paraspinal neurofibromas than in those without paraspinal tumors. »: Patients with NF1 are at low risk for developing malignant peripheral nerve sheath tumors during childhood (<1%) and their lifetime (8% to 12%), and routine imaging surveillance for malignancy in the absence of symptoms should be clinically directed. »: Further investigation is needed to standardize screening for EOS in children with NF1 and to develop guidelines for ideal imaging modalities, including their frequency and a timeline.

Original languageEnglish (US)
JournalJBJS reviews
Volume9
Issue number7
DOIs
StatePublished - Jul 23 2021

ASJC Scopus subject areas

  • Surgery
  • Orthopedics and Sports Medicine

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